title: [Bullous pemphigoid: a review].
abstract: BACKGROUND
  Bullous pemphigoid (BP) is the most common auto-immune bullous disorder. Its treatment is difficult due to high age and comorbidities of affected patients.
  
  
  OBJECTIVE
  To assess the effects of treatments for BP.
  
  
  METHODS
  Randomized therapeutic trials (RCTs) were identified using an automatic search on Pubmed et Embase until March 2009. Large retrospective series with homogeneous therapeutic management were also selected and analyzed.
  
  
  RESULTS
  Forty-four articles were selected and analyzed, which included nine RCTs with a total of 1007 participants (653 patients were included in two trials). Two RCTs comparing different modalities of systemic corticosteroid therapy failed to show differences in measure of disease control. The addition of plasma exchanges (one RCT) or azathioprine (one RCT) allowed to halve the amount of prednisone required for disease control. A further 3-arms RCT compared plasma exchange or azathioprine plus prednisone, but failed to show significant differences for disease control or mortality of BP. One study compared tetracycline plus nicotinamide with prednisolone, no significant difference for disease response was evidenced. A large controlled clinical trial demonstrated that high doses of very potent topical corticosteroids increased initial disease control and 1-year survival of patients with extensive BP, as compared with oral prednisone. Another RCT compared two regimens of potent topical corticosteroids and a non-inferior rate of BP control was obtained with the mild regimen. Finally, a study comparing two immunosuppressant drugs (azathioprine, mycophenolate mofetil) in addition to prednisone failed to show any difference for disease control, recurrence rate or the cumulated doses of prednisone.
  
  
  CONCLUSIONS
  Ultrapotent topical corticosteroids (clobetasol propionate; 20 to 40g/day) are effective treatments for BP with fewer systemic side-effects than oral high-dose corticosteroids. Systemic corticosteroids are effective but doses greater than 0.5mg/kg per day are associated with severe side-effects, including decreased survival. The effectiveness of the addition of plasma exchange or immunosuppressants (azathioprine, mycophenolate mofetil) to systemic corticosteroids has not been established. Combination treatment with tetracycline and nicotinamide needs further validation.
authors.affiliation: Service de dermatologie, CHU de Reims, 47 rue Serge-Kochman, Reims, France. pbernard@chu-reims.fr
authors.forename: P
authors.initials: P
authors.lastname: Bernard
authors.forename: J
authors.initials: J
authors.lastname: Charneux
mesh_terms: D000368:Aged; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D002990:Clobetasol / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003622:Dapsone / Q000008:administration & dosage / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015201:Meta-Analysis as Topic; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D009536:Niacinamide / Q000008:administration & dosage / Q000627:therapeutic use; D010391:Pemphigoid, Bullous / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D010951:Plasma Exchange; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic / Q000706:statistics & numerical data; D012008:Recurrence; D012189:Retrospective Studies; D013752:Tetracycline / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D009536:Niacinamide; D003622:Dapsone; D002990:Clobetasol; D013752:Tetracycline; D009173:Mycophenolic Acid; D001379:Azathioprine; D011241:Prednisone